|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Resveratrol treatment reduces the vulnerability of SH-SY5Y cells and cortical neurons overexpressing SOD1-G93A to Thimerosal toxicity through SIRT1/DREAM/PDYN pathway.
|
30503815 |
2019 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
BEFREE |
The neurotoxin beta-N-methylamino-l-alanine (BMAA) was first identified as a "toxin of interest" in regard to the amyotrophic lateral sclerosis-Parkinsonism Dementia Complex of Guam (ALS/PDC); studies in recent years highlighting widespread environmental sources of BMAA exposure and providing new clues to toxic mechanisms have suggested possible relevance to sporadic ALS as well.
|
24918341 |
2014 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Lack of synergistic effect of resveratrol and sigma-1 receptor agonist (PRE-084) in SOD1G⁹³A ALS mice: overlapping effects or limited therapeutic opportunity?
|
24885036 |
2014 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Identification of a misfolded region in superoxide dismutase 1 that is exposed in amyotrophic lateral sclerosis.
|
25164820 |
2014 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Motor neuron dysfunction in a mouse model of ALS: gender-dependent effect of P2X7 antagonism.
|
23583883 |
2013 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Bromocriptine methylate suppresses glial inflammation and moderates disease progression in a mouse model of amyotrophic lateral sclerosis.
|
21867702 |
2011 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Disrupted zinc-binding sites in structures of pathogenic SOD1 variants D124V and H80R.
|
20515040 |
2010 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Transcriptional profiling in the lumbar spinal cord of a mouse model of amyotrophic lateral sclerosis: a role for wild-type superoxide dismutase 1 in sporadic disease?
|
20177826 |
2010 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Mutant SOD1 knockdown in all cell types ameliorates disease in G85R SOD1 mice with a limited additional effect over knockdown restricted to motor neurons.
|
20132483 |
2010 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Rapid one-step purification of native dimeric ALS-associated human Cu/Zn superoxide dismutase from transgenic rat tissues.
|
19929749 |
2010 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
From transcriptome analysis to therapeutic anti-CD40L treatment in the SOD1 model of amyotrophic lateral sclerosis.
|
20348957 |
2010 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
A common property of amyotrophic lateral sclerosis-associated variants: destabilization of the copper/zinc superoxide dismutase electrostatic loop.
|
19635794 |
2009 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Superoxide dismutase 1 mutants related to amyotrophic lateral sclerosis induce endoplasmic stress in neuro2a cells.
|
18233996 |
2008 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Identification and characterization of cholest-4-en-3-one, oxime (TRO19622), a novel drug candidate for amyotrophic lateral sclerosis.
|
17496168 |
2007 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Metallothionein proteins expression, copper and zinc concentrations, and lipid peroxidation level in a rodent model for amyotrophic lateral sclerosis.
|
17097207 |
2007 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Protein-bound crotonaldehyde accumulates in the spinal cord of superoxide dismutase-1 mutation-associated familial amyotrophic lateral sclerosis and its transgenic mouse model.
|
17319283 |
2007 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2-21.3.
|
16495328 |
2006 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
GeneticVariation
|
disease |
BEFREE |
A family history of ALS/PDC was recorded in more than 70% of patients, but no abnormal mutation or polymorphism was found in the genes of SOD1, tau, and apolipoprotein E. Familial nature and continuing morbidity of Kii ALS/PDC suggest that genetic factors may be more likely in its pathogenesis.
|
16092099 |
2005 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Glycation proceeds faster in mutated Cu, Zn-superoxide dismutases related to familial amyotrophic lateral sclerosis.
|
12626432 |
2003 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
A therapeutic role for cyclooxygenase-2 inhibitors in a transgenic mouse model of amyotrophic lateral sclerosis.
|
12586733 |
2003 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
BEFREE |
The lack of mutations in the tau gene not only in this patient but also in earlier reported cases of ALS in the Western Pacific seems to suggest that other genetic factors may be contributing to ALS/PDC.
|
12899197 |
2003 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Oxidative stress causes abnormal accumulation of familial amyotrophic lateral sclerosis-related mutant SOD1 in transgenic Caenorhabditis elegans.
|
11590119 |
2001 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Increased expression of the pro-inflammatory enzyme cyclooxygenase-2 in amyotrophic lateral sclerosis.
|
11220737 |
2001 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Transgenic SOD1 G93A mice develop reduced GLT-1 in spinal cord without alterations in cerebrospinal fluid glutamate levels.
|
11723166 |
2001 |
|
Amyotrophic Lateral Sclerosis, Guam Form
|
0.330 |
Biomarker
|
disease |
CTD_human |
Increased reactive oxygen species in familial amyotrophic lateral sclerosis with mutations in SOD1.
|
10930589 |
2000 |